He Guanwen, Wei Rifu, Chen Liwen, Hong Yiyun, Li Huifeng, Liu Ping, Xu Yangbin, Zhu Zhongshou
Department of Otolaryngology, Ningde Municipal Hospital Affiliated of Ningde Normal College (Ningde Institute of Otolaryngology), Ningde, 352100, Fujian, China.
Eur Arch Otorhinolaryngol. 2022 Apr;279(4):1787-1794. doi: 10.1007/s00405-021-06851-0. Epub 2021 May 4.
Limited literature exists on primary external auditory canal (EAC) cholesteatoma (EACC). Here, we focus on the clinical features of this rare disease, especially the invasive patterns of lesion progression, through a large population study and present simple and practical staging.
In all, 276 patients (male 99; female 177; mean age 41.3 ± 21 years; ears 301) with primary EACC were retrospectively analyzed. Stage I indicated EACC without bony lesions, stage II indicated invasion confined within EAC, stage III indicated invasion beyond the EAC involving mastoid air cells or tympanic cavity, but within the temporal bone, and stage IV indicated invasion beyond the temporal bone.
In all, 41, 219, 40, and 1 ear with Stage I, II, III, and IV lesions were found, respectively. Common clinical symptoms were hearing loss (237 ears, 78.7%), otalgia (221 ears, 73.4%), and otorrhea (85 ears, 28.2%). The mean air conduction and air-bone gaps were 45.4 ± 17.9 dB HL and 24.6 ± 15 dB HL, respectively. EACCs were found to invade in all directions of the EAC, with the inferior wall (224 ears, 74.4%) > posterior wall (207 ears, 68.8%) > anterior wall (186 ears, 61.8%) > superior wall (86 ears, 28.6%) invasion; multiwall invasions (207 ears) were common; however, inward invasions into the tympanic cavity were rare.
Primary EACCs occurred mostly in women and often unilaterally invaded multiple bony walls in the lower half of the EAC. The present staging reflects the patterns and severity of lesion progression and may be beneficial in treatment planning.
关于原发性外耳道胆脂瘤(EACC)的文献有限。在此,我们通过一项大规模人群研究,聚焦于这种罕见疾病的临床特征,尤其是病变进展的侵袭模式,并提出简单实用的分期方法。
对276例原发性EACC患者(男性99例;女性177例;平均年龄41.3±21岁;患耳301只)进行回顾性分析。I期表示无骨质病变的EACC,II期表示局限于外耳道内的侵袭,III期表示超出外耳道累及乳突气房或鼓室,但在颞骨内,IV期表示超出颞骨的侵袭。
分别发现I、II、III和IV期病变的患耳为41、219、40和1只。常见临床症状为听力损失(237耳,78.7%)、耳痛(221耳,73.4%)和耳漏(85耳,28.2%)。平均气导和骨气导差分别为45.4±17.9dB HL和24.6±15dB HL。发现EACC向外耳道各方向侵袭,其中下壁侵袭(224耳,74.4%)>后壁侵袭(207耳,68.8%)>前壁侵袭(186耳,61.8%)>上壁侵袭(86耳,28.6%);多壁侵袭(207耳)常见;然而,向内侵袭至鼓室的情况罕见。
原发性EACC大多发生于女性,常单侧侵袭外耳道下半部的多个骨壁。目前的分期反映了病变进展的模式和严重程度,可能有助于治疗方案的制定。